The Fontan Pathway: Change in Dimension and Catheter-Based Intervention over Time.

An unobstructed Fontan pathway is essential for optimal hemodynamics. We hypothesize that more extracardiac conduit (ECC) Fontan pathways develop obstruction compared to lateral tunnel (LT) Fontans and that the dilation typically observed in LTs results in similar mid-term clinical outcomes. A single-center, retrospective study was done including all Fontan cardiac catheterizations from 2006 to 2019. Angiography and medical records were reviewed to define Fontan pathway dimensions, interventions, and clinical outcomes. 232 patients underwent cardiac catheterization, where 60% were ECCs and 30% LTs. The minimum cross-sectional area (CSA) of ECCs was significantly smaller than LTs and LTs dilated over time. 13% of patients had Fontan pathway stenting at a median age of 16.2 years. The minimum CSA for patients who underwent intervention was significantly smaller than patients who did not. Lower weight at Fontan surgery was associated with intervention on the Fontan pathway, with a threshold weight of 15 kg for patients with an ECC. The median follow-up was 3.3 years. Patients who had Fontan pathway intervention were not more likely to experience the composite adverse clinical outcome. LTs were more likely than ECCs to have worse clinical outcome, when liver fibrosis was included. This is the first study to describe angiographic dimensions of the Fontan pathway in a large number of patients over time. ECCs tend to become stenotic. Lower weight at Fontan surgery is a potential risk for Fontan pathway intervention. LTs may experience worse clinical outcomes in follow-up. This information can help inform the optimal timing and method of post-Fontan surveillance.

Arrhythmias After Fontan Operation with Intra-atrial Lateral Tunnel Versus Extra-cardiac Conduit: A Systematic Review and Meta-analysis.

Current studies on the incidence of arrhythmias after the intra-atrial lateral tunnel (ILT) Fontan operation and the extra-cardiac conduit (ECC) Fontan operation are limited, with controversial results. This systematic review aimed to compare the prevalence of arrhythmias in patients who underwent ECC or ILT Fontan. Relevant studies comparing the incidence of arrhythmias and pacemaker implantation in ILT with ECC were identified through a literature search using MEDLINE, EMBASE, and the cochrane central register of controlled trials. The outcome measures included baseline characteristics, early (≤30 days) and late (>30 days) arrhythmias and pacemaker implantation. 16 publications involving 3499 patients were included. In the meta-analysis, although the overall risk of early arrhythmias was lower for the ILT group, statistically, no significant difference was observed (odds ratio [OR] 0.78; 95% confidence interval [CI] 0.61-1.01; p = 0.06). Similarly, there was no significant difference between the two cohorts in the incidence of postoperative permanent pacemaker therapy (OR 1.36; 95% CI 0.86-2.14; p = 0.19). However, we found significantly increased incidence of late arrhythmias in ILT group compared with ECC group (OR 1.96; 95% CI 1.64-2.35; p < 0.01). Although our systematic review and meta-analysis suggested that there was no significant difference in early arrhythmias and in pacemaker implantation between the ILT and ECC groups, ECC procedure could significantly lower the risk of late arrhythmias after Fontan surgery. Given that some limitations cannot be overcome, well-designed randomized controlled trials are needed to confirm our findings.

Does the External Pericardial Lateral Tunnel Fontan Pathway Enlarge to Accommodate Somatic Growth? A Preliminary Analysis.

Objectives: An ideal Fontan pathway should be capable of adapting to changes in circulatory demands. The external pericardial lateral tunnel Fontan (EPLTF) is constructed of viable, autologous tissue and may be capable of changing in size. We investigated the ability of the EPLTF to enlarge with increasing physiologic demands of somatic growth. Methods: Retrospective review of echocardiographic images for patients with a minimum of five years follow-up after EPLTF. Serial echocardiographic measurements of the EPLTF pathway were obtained at three distinct locations: the inferior vena cava junction with the EPLTF, midsection of the EPLTF, and cross-sectional area of the EPLTF visualized in a four-chamber view. Body surface area (BSA) was calculated at the time of each echocardiographic measurement. Changes in echocardiographic measurements over time were analyzed and compared with changes in BSA. Results: A total of 332 echocardiographic studies from 38 patients were reviewed. Significant enlargement of the EPLTF pathway is observed at the inferior vena caval junction (P < .001), midsection (P < .01), and cross-sectional area (P < .001). Repeated measures correlation between pathway measurements and BSA is highly significant (P < .001). Conclusions: The EPLTF pathway enlarges over time in correlation with increasing BSA. Further research is needed to define ideal pathway size, differentiate normal physiologic growth from pathologic enlargement, and correlate changes with clinical outcomes.

Alarming rate of liver cirrhosis after the small conduit extracardiac Fontan: A comparative analysis with the lateral tunnel.

BACKGROUND: The association between the prevalence of cirrhosis and the types of Fontan operations remains unclear. METHODS: We conducted a retrospective chart review of 332 patients who underwent the Fontan procedure at our institution. Four patients who underwent the atriopulmonary connection Fontan were excluded from the analysis. Patients who had intracardiac-extracardiac conduit (126) between 1989 and 2021 were pooled with those having extracardiac conduit (ECC) (134). The 260 patients who underwent the ECC and the 68 patients who had the lateral tunnel (LT) Fontan constitute the core of the study. RESULTS: Median age at the Fontan procedure was 23.7 months (interquartile range [IQR], 20.8-32.6) in the LT group, compared with 28.8 months (IQR, 24.6-39.5) in the ECC group (P < .01). The median follow-up was 14.8 years (IQR, 12.5-16.5) in the LT group and 7 years (IQR, 2.8-10.4) in the extracardiac conduit group. During the follow-up period, 3 patients (4.4%) with LT and 17 patients (6.5%) with ECC (11 patients with 16 mm or less conduit size) were diagnosed with cirrhosis. The prevalence of cirrhosis at 1, 5, 10, and 15 years was 0%, 0%, 0%, and 4.4% in the LT group, respectively, and 0%, 0.9%, 7.7%, and 29.8% in the ECC group (P < .01) Rates of mortality, Fontan revision, Fontan takedown, transplant, and complications were comparable between the 2 groups. CONCLUSIONS: The extracardiac conduit Fontan seems to be associated with faster development of cirrhosis.

Biomechanical Analysis of Age-Dependent Changes in Fontan Power Loss.

The hemodynamics in Fontan patients with single ventricles rely on favorable flow and energetics, especially in the absence of a subpulmonary ventricle. Age-related changes in energetics for extracardiac and lateral tunnel Fontan procedures are not well understood. Vorticity (VOR) and viscous dissipation rate (VDR) are two descriptors that can provide insights into flow dynamics and dissipative areas in Fontan pathways, potentially contributing to power loss. This study examined power loss and its correlation with spatio-temporal flow descriptors (vorticity and VDR). Data from 414 Fontan patients were used to establish a relationship between the superior vena cava (SVC) to inferior vena cava (IVC) flow ratio and age. Computational flow modeling was conducted for both extracardiac conduits (ECC, n = 16) and lateral tunnels (LT, n = 25) at different caval inflow ratios of 2, 1, and 0.5 that corresponded with ages 3, 8, and 15+. In both cohorts, vorticity and VDR correlated well with PL, but ECC cohort exhibited a slightly stronger correlation for PL-VOR (>0.83) and PL-VDR (>0.89) than that for LT cohort (>0.76 and > 0.77, respectively) at all ages. Our data also suggested that absolute and indexed PL increase (p < 0.02) non-linearly as caval inflow changes with age and are highly patient-specific. Comparison of indexed power loss between our ECC and LT cohort showed that while ECC had a slightly higher median PL for all 3 caval inflow ratio examined (3.3, 8.3, 15.3) as opposed to (2.7, 7.6, 14.8), these differences were statistically non-significant. Lastly, there was a consistent rise in pressure gradient across the TCPC with age-related increase in IVC flows for both ECC and LT Fontan patient cohort. Our study provided hemodynamic insights into Fontan energetics and how they are impacted by age-dependent change in caval inflow. This workflow may help assess the long-term sustainability of the Fontan circulation and inform the design of more efficient Fontan conduits.

27 years of experience with the Fontan procedure: characteristics and clinical outcomes of children in a tertiary referral hospital.

BACKGROUND: The Fontan operation has improved the survival of children born with congenital heart disease with single ventricle physiology. The most widely adopted variations of the Fontan procedure are the extracardiac conduit, the lateral tunnel ve the intra/extracardiac conduit with fenestration. Despite advances in the treatment and prevention of early and late complications that may develop after Fontan surgery, morbidity still remains an important problem. METHODS: 304 patients who underwent Fontan surgery in our center between 1995 and 2022 were included in our study. The complications that developed in patients who underwent primary Fontan or lateral tunnel surgery and extracardiac conduit Fontan application were compared. RESULTS: Classic Fontan surgery and lateral tunnel surgery were performed in 26 of the patients, and extracardiac Fontan surgery was performed in 278 patients. 218 of 304 cases were patients with single ventricular pathology. 86 cases were patients with two ventricular morphologies but complex cardiac pathology. Fenestration was performed in only 6 patients, other patients did not require fenestration. The mean follow-up period of our patients was 12 years (3 months-27 years). When the complications between Fontan procedures were compared in our study, it was found that the length of hospital stay and mortality were statistically significantly reduced in patients who underwent extracardiac Fontan surgery. There was no significant difference in terms of complications that can be seen after Fontan surgery and the length of stay in the intensive care unit. CONCLUSION: Fontan complex is a palliative surgery for children with complex heart disease. Palliative surgical operations aimed at the preparation of the Fontan circulation lead to the preparation of the pulmonary vascular bed and the preservation of ventricular function. The techniques applied in Fontan surgery affect the early and long-term complications and the survival of the patients. In our study, when we examined the patients who extracardiac conduit Fontan procedure for the non-cardiac route, we found that mortality and morbidity were minimal.

Late development of intra-atrial reentrant tachycardia in lateral tunnel Fontan patients and the preventive role of prophylactic cryoablation.

OBJECTIVE: Intra-atrial reentrant tachycardia is an important late-onset complication in patients undergoing the Fontan procedure. However, the protective effects of prophylactic cryoablation against late-onset intra-atrial reentrant tachycardia are unclear. This study investigated the late development of intra-atrial reentrant tachycardia in patients undergoing the lateral tunnel Fontan procedure and the role of prophylactic cryoablation. METHODS: This was a single-center retrospective cohort study of patients who underwent the lateral tunnel Fontan procedure between 1988 and 2003. Univariate and multivariable competing risks regression models were used to determine the associations of prophylactic cryoablation and covariates with the outcomes of interest: late-onset intra-atrial reentrant tachycardia, all-cause mortality, and cardiovascular mortality. RESULTS: In total, 130 patients who underwent the lateral tunnel Fontan procedure, 30 of whom had undergone prophylactic cryoablation, were included in this study and followed up for a median of 23.6 years (interquartile range, 17.7-26.5). Intra-atrial reentrant tachycardia was identified in 14 patients (10.8%), none of whom underwent prophylactic cryoablation. The median Fontan-to-intra-atrial reentrant tachycardia time was 17.2 years (interquartile range, 11.1-23.1). Prophylactic cryoablation was protective against late-onset intra-atrial reentrant tachycardia (P < .0001) and cardiovascular mortality (P < .0001) in the type 3 test. CONCLUSIONS: None of the patients who underwent prophylactic cryoablation developed late-onset intra-atrial reentrant tachycardia during a median follow-up time of 22.9 years. Our study demonstrated that prophylactic cryoablation was effective in preventing late-onset intra-atrial reentrant tachycardia and cardiovascular mortality in patients undergoing the lateral tunnel Fontan.

The Epidemiology of Persons Living with Fontan in 2020 and Projections for 2030: Development of an Epidemiology Model Providing Multinational Estimates.

INTRODUCTION: Fontan surgery is a palliative procedure performed in children with a functionally univentricular heart. Improvements in surgical technique over the past 30 years have increased life expectancy in this rare population. However, the epidemiology of persons living with Fontan is poorly understood. This study aimed to estimate the 2020 and 2030 prevalence of persons living with a Fontan circulation in 11 countries across the US, Europe, Australia and New Zealand, by procedure type: [atriopulmonary connection (AP), lateral tunnel total cavopulmonary connection (LT-TCPC) or extracardiac total cavopulmonary connection (EC-TCPC)]; and age group: [children (< 12 years), adolescents (12-17 years), and adults (≥ 18 years old)] by building an epidemiologic model. METHODS: The annual number of Fontan surgeries by country in 2010-2020 were extracted from hospital or claims databases, via procedure codes. The epidemiology of persons living with Fontan was modelled by applying these surgery frequencies to mid-year populations from 1972 to 2020 and overlaying an uptake curve. A literature search identified: 30-day mortality rates, long-term survival, and median age at surgery. Averages of these estimates were inputted into the model to project prevalence in 2030. RESULTS: The number of persons living with Fontan in 2020 across the 11 countries was estimated to be 47,881 [66 people per million (ppm)], rising to 59,777 (79 ppm) by 2030. In 2020, this population was 55% adults, 17% adolescents and 28% children shifting to 64%, 13% and 23%, respectively, in 2030. Among all persons living with Fontan, 74%/18%/9% are estimated to have EC-TCPC/LT-TCPC/AP, respectively, in 2020, and 83%/14%/4% in 2030. CONCLUSIONS: According to this epidemiology model, the Fontan population is growing, partly driven by increased survival rates with the more recent LT-TCPC and EC-TCPC procedures (compared with AP). The 2020/2030 prevalence of persons living with Fontan is 66/79 ppm.

Fontan with lateral tunnel is associated with improved survival compared with extracardiac conduit.

OBJECTIVES: The study aim was to compare Fontan patients undergoing lateral tunnel (LT) versus extracardiac conduit (ECC) technique. METHODS: Fontan patients (LT vs ECC) from January 2000 to December 2017 were analyzed retrospectively. Baseline characteristics were analyzed as covariates. Primary outcomes (ie, mortality, Fontan failure, thrombosis, and pacemaker implantation) were compared using time-to-event models. Subgroup analysis including only initially fenestrated cases and propensity score matching were performed. RESULTS: Eight hundred one Fontan patients: LT (n = 638) versus ECC (n = 163) were included. Median follow-up time was 4.8 years (range, 1.1-10.8 years). Baseline characteristics were similar except for age: LT versus ECC: 2.6 years (range, 2.2-3.2 years) versus 3.1 years (range, 2.6-4.7 years) (P < .01) and mean pulmonary artery pressure: LT versus ECC: 12 mm Hg (11-15 mm Hg) versus 11 mm Hg (10-13 mm Hg) (P < .05). Early mortality was significantly higher in ECC versus LT group (3.1%; vs 0.5%; P < .05). Freedom from death, heart transplantation and Fontan failure were significantly longer in LT vs ECC (P < .01). After correcting for age, diagnosis, surgical technique, surgeon, mean pulmonary artery pressure, and fenestration, the ECC group showed worse freedom from death (hazard ratio, 2.8; P < .01) and Fontan failure (hazard ratio, 3.0; P < .01). No difference in pacemaker implantation rate was demonstrated (P = .25). Early fenestration closure was associated with higher risk of early (hazard ratio, 30.5) and late mortality (hazard ratio, 3.5). After matching, log-rank tests showed significant differences between the 2 groups for Fontan failure at 5 and 10 years (P < .01) and mortality at 5 years (P = .02). CONCLUSIONS: When compared with ECC, LT Fontan is associated with better short and midterm outcomes. Spontaneous fenestration closure is an independent risk factor for early/late mortality.

Lateral tunnel Fontan atrial tachycardia ablation trans-baffle access is not mandatory as the initial strategy.

BACKGROUND: Mapping and ablation of atrial tachycardia (AT) is commonly performed in lateral tunnel Fontan (LTF) patients, yet there is little information on the need of baffle puncture to access the pulmonary venous atrium (PVA). This study aimed to evaluate the most common chamber location of critical sites for majority of AT in LTF patients. METHODS: Consecutive LTF patients underwent catheter-based high-density mapping and ablation of AT from Nov. 2015 to Mar. 2019. Critical sites were identified by a combination of activation and entrainment mapping. Acute procedural success was defined as AT termination with ablation and non-inducibility of any AT. Predictors for ablation failure were evaluated in retrospect. RESULTS: Fifteen catheter ablation procedures were performed in 9 patients. A total of 15 clinical ATs (mean TCL 369 ± 91 ms) were mapped. The mechanism was macro re-entry in 11 (73%) and micro re-entry in 2. In 11 ATs (73%), 94 ± 5% of tachycardia cycle length (TCL) were mapped inside the tunnel. The commonest site of successful ablation in the tunnel was on the lateral wall (60%). Trans-baffle access was obtained during 5 of 15 procedures (33%). Overall, procedural success was achieved in 9 of 15 procedures (60%). There were no complications. Recurrence of AT was 42% over a follow-up period of 4.3 ± 3.2 years. Faster TCL of 200-300 ms showed a trend towards ablation failure, (OR 17, 95% CI 0.7 to 423, p = 0.08). CONCLUSIONS: Catheter ablation can be performed effectively for ATs in LTF patients usually from inside the tunnel. ATs with critical sites in the PVA are uncommon. This information will help plan ablation in LTF patients without resorting to initial trans-baffle access.

Ultra-high density mapping of intra-atrial reentrant tachycardia in a patient after a lateral tunnel total cavopulmonary connection.

We report a case of an 18-year-old male with a postsurgical lateral tunnel (LT) total cavopulmonary connection (TCPC) and supraventricular tachycardia (SVT). Patients after an LT TCPC have complicated suture lines and a considerable area of damaged myocardium in the LT, which could become a complex arrhythmogenic substrate of tachycardias. Detailed three-dimensional (3D) mapping of the LT and atrium is important for a successful ablation. In this patient, successful catheter ablation of two types of complex tachycardias was accomplished using an ultra-high density 3D mapping system inside the LT.

Progressive loss of bone mass in children with Fontan circulation.

OBJECTIVE: We investigated bone mineral density (BMD) at different ages after the Fontan completion, and we evaluated the relationship between BMD, vitamin D levels, and pertinent patient variables. METHODS: A cross-sectional sample of 64 patients was examined with dual-energy X-ray absorptiometry (DXA) scans to determine BMD. Of these patients, 24 were also examined with BoneXpert software to determine bone mass density (BMX), expressed as the bone health index (BHI). Blood samples from all patients were analyzed. Patients were divided into three different age groups; A: 4-9 years old (n = 22), B: 10-15 years old (n = 21), and C: 16-18 years old (n = 21). RESULTS: Overall, BMD z scores were (mean ± SD): -1.0 ± 1.3 for the lumbar spine and -0.2 ± 1.2 for the total body. Groups B and C had significantly lower z score values compared to group A. Of patients in group C, 35% had z score values ≤-2 SD of the mean of the healthy population. There was no difference related to systemic ventricular anatomy (left or right); however, patients with lateral tunnels had lower BMD than patients with extra cardiac conduits. Overall, the BHI z score was (mean ± SD): -1.2 ± 0.9, but low BMX did not correlate with low BMD. The 25-hydroxy vitamin D level was 58 ± 30 nmol/L. Vitamin D levels decreased with age: in group C, 33.3% of patients exhibited vitamin D deficiencies. Vitamin D levels were not correlated with bone mineral densities. CONCLUSION: BMD levels decreased with age in patients with Fontan circulation. Different bone components were involved. Vitamin D levels also decreased with age, but they were not consistently associated with bone mineral densities. The single factor most predictive of low BMD was a lateral tunnel Fontan, compared to an extra cardiac Fontan.

Staged total cavopulmonary connection: serial comparison of intra-atrial lateral tunnel and extracardiac conduit taking account of current surgical adaptations.

OBJECTIVES: Our goals were to compare the outcome of the intra-atrial lateral tunnel (ILT) and the extracardiac conduit (ECC) techniques for staged total cavopulmonary connection (TCPC) and to compare the current modifications of the TCPC technique, i.e. the prosthetic ILT technique with the current ECC technique with a ≥18-mm conduit. METHODS: We included patients who had undergone a staged TCPC between 1988 and 2008. Records were reviewed for patient demographics, operative details and events during follow-up (death, surgical and catheter-based reinterventions and arrhythmias). RESULTS: Of the 208 patients included, 103 had the ILT (51 baffle, 52 prosthetic) technique and 105 had the ECC technique. Median follow-up duration was 13.2 years (interquartile range 9.5-16.3). At 15 years after the TCPC, the overall survival rate was comparable (81% ILT vs 89% ECC; P = 0.12). Freedom from late surgical and catheter-based reintervention was higher for patients who had ILT than for those who had ECC (63% vs 44%; P = 0.016). However, freedom from late arrhythmia was lower for patients who had ILT than for those who had ECC (71% vs 85%, P = 0.034). In a subgroup of patients who had the current TCPC technique, when we compared the use of a prosthetic ILT with ≥18-mm ECC, we found no differences in freedom from late arrhythmias (82% vs 86%, P = 0.64) or in freedom from late reinterventions (70% vs 52%, P = 0.14). CONCLUSIONS: A comparison between the updated prosthetic ILT and current ≥18-mm ECC techniques revealed no differences in late arrhythmia-free survival or late reintervention-free survival. Overall, outcomes after the staged TCPC were relatively good and reinterventions occurred more frequently in the ECC group, whereas late arrhythmias were more common in the ILT group.

Comparison of extracardiac conduit and lateral tunnel for functional single-ventricle patients: A meta-analysis.

OBJECTIVE: This study aims to assess and compare the early and long-term effects of extracardiac conduit (EC) and lateral tunnel (LT) in patients with a functional single ventricle through meta-analysis. DESIGN: A systematic search was performed in PubMed, Embase, Cochrane Library, CNKI, VIP, CBM, and WanFang databases for papers that were published until August 1, 2016. Cochrane systematic review method was used for paper screening and information retrieve, and RevMan 5.3 software was applied for the meta-analysis. RESULTS: Data for 10 studies with a total of 3814 patients were retrieved. The advantages of EC comparing to LT include: lower 30 day postsurgery supraventricular arrhythmia incidence (Relative Risk [RR] = 0.31 [0.17, 0.55], P < .001), lower protein loss enteropathy incidence (RR = 0.33 [0.11, 0.96], P = .04), and requiring no cardiopulmonary bypass. However, the chest drainage time was longer (mean difference [MD] = 1.99 [1.83, 2.15], P < .001) in EC. There were no significant differences in early postoperative mortality, long-term mortality, long-term arrhythmia, Fontan takedown, ventilator-assisted ventilation, ICU stay, thrombosis, pleural effusion, and pericardial effusion between EC and LT. CONCLUSIONS: EC had a lower incidence of supraventricular arrhythmia (30 days after operation) and the rate of protein losing enteropathy than LT, and requiring no cardiopulmonary bypass. These show that EC has an advantage over the LT in patients with a functional single ventricle.

Sinus Node Dysfunction After Extracardiac Conduit and Lateral Tunnel Fontan Operation: The Importance of the Type of Prior Superior Cavopulmonary Anastomosis.

This review summarizes the influence of the type of prior superior cavopulmonary anastomosis in the studies that report a difference in the occurrence of sinus node dysfunction among extracardiac conduit Fontan and lateral tunnel Fontan operations along with clinical implications.

Creating a Fontan fenestration in a child with dextrocardia and interrupted inferior vena cava.

Plastic bronchitis is a rare life-threatening complication of the Fontan operation. Transcatheter Fontan fenestration can ameliorate symptoms by decompressing elevated venous pressures. Transcatheter creation of a fenestration can be technically challenging in cases with complex venous anatomy. We report a case of a 5-year-old boy with heterotaxy, dextrocardia with unbalanced atrioventricular canal (AVC), atrial and visceral situs inversus, left-sided superior vena cava (SVC), and left-sided interrupted inferior vena cava (IVC) with azygos continuation. With few modifications to the equipment, a successful Fontan fenestration with stent implantation was performed via transjugular approach. At 2-year follow-up, his symptoms of plastic bronchitis improved significantly.

Thrombosis and thromboembolic complications in fontan patients: a literature review.

Hemodynamic fluctuations and thromboembolic complications are significant areas of concern during the postoperative management of patients with univentricular hearts. The objective of this study is to review the incidence and risk factors associated with thrombosis and thromboembolic complications following total cavopulmonary anastomosis, the third stage of the palliative surgical procedure. A literature search of published evidence was conducted on OvidSP MEDLINE(R) and Embase followed by paired title, abstract, and full-text screening based on specific inclusion criteria. High risks of thromboembolic outcomes were identified across studies, with variable incidences between 3% and 20%, high mortality rates up to 38%, and an inverse relationship with prophylaxis treatment administration. Several risk factors for thrombotic complications, including chronic systemic venous hypertension, protein-losing enteropathy, passive blood flow, atrial arrhythmias, conduit stenosis, prosthetic material use, coagulation factor abnormalities, and several patient characteristics were identified. Based on these findings, a prophylactic anticoagulation algorithm has been proposed.

Anatomical and functional assessment of the intra-atrial lateral tunnel in the Fontan circulation.

OBJECTIVES: In patients after completion of the total cavopulmonary connection (TCPC) with an intra-atrial lateral tunnel, deviations of the tunnel from an ideal straight tubular shape were observed. However, little is known about frequency and adverse effects of such shape deviations. We sought to analyse tunnel anatomy, dimensions and blood flow using cardiac magnetic resonance imaging (CMR). METHODS: Fifty-four patients with hypoplastic left heart syndrome (HLHS; mean age 6.0 ± 2.4 years) underwent CMR with gradient-echo cine sequences, 2D- and 3D-phase-contrast imaging. We analysed anatomy, diameters, cross-sectional areas, volumes and blood flow of the tunnel. RESULTS: Twenty-five patients had a tubular tunnel. In 29 patients, bulging and/or narrowing of the tunnel were present. Cross-sectional areas and volumes of the tunnel were not significantly different between the two groups. There were also no differences for the mean blood flow and the mean and maximal flow velocity (P = 0.05-0.6). In all the patients, the normalized tunnel volume was related to age (r = 0.44; P = 0.002) and body surface area (BSA; r = 0.42; P = 0.005). The mean tunnel blood flow correlated with age (r = 0.73; P = 0.001) and BSA (r = 0.83; P < 0.0001). CONCLUSIONS: A considerable percentage of patients with an intra-atrial lateral tunnel develop mild deviations of the tunnel from ideal tubular shape. The correlation between tunnel volume and mean blood flow with age and BSA suggests that the capacity of the tunnel adjusts to body growth, independent of tunnel shapes that deviate from a fluid-dynamically favourable shape. Follow-up CMRs are needed to detect long-term effects of irregular tunnel shapes on flow dynamics.